Externally applied corticosteroids increase the intraocular pressure in one-third of the general population. We evaluated the intraocular pressure in 62 patients with endogenously elevated serum cortisol levels due to hypothalamic pituitary-dependent Cushing's disease. All patients underwent transphenoidal microsurgical sella exploration within 4 years after the onset of subjective symptoms. The intraocular pressure (preoperative mean, 14.9 +/- 3.5 mm Hg) exceeded 21 mm Hg in four eyes before surgery and in one eye after surgery (postoperative mean, 14.3 +/- 2.8 mm Hg). In patients attaining postoperative remission and whose pressure readings were taken at least 3 months after surgery, the postoperative intraocular pressure was significantly (P = 0.04) lower than that measured preoperatively (14.2 +/- 2.7 vs 13.0 +/- 2.0 mm Hg). Biomorphometry of the optic disc topography and the retinal nerve-fiber layer in 19 patients revealed no glaucomatous changes. In contrast to topically applied corticosteroids, endogenously elevated serum cortisol levels in patients with hypothalamic pituitary-dependent Cushing's disease increased intraocular pressure only slightly, with no great risk of glaucomatous optic nerve damage.
Seventeen optic disks with morning glory syndrome were measured in absolute and relative size units (millimeters and parapapillary retinal vessel caliber) in 5 and 12 eyes, respectively. Their respective mean areas were 7.47 +/- 2.63 mm2 and 7.84 +/- 4.96 mm2. They were significantly (Mann-Whitney test) larger than 457 unselected normal optic nerve heads (2.69 +/- 0.70 mm2; p less than 0.0001) and 15 optic disks with pits (4.84 +/- 1.42 mm2; p less than 0.001). They are the largest optic nerve heads, probably with constant postnatal size, measured so far, and form part of a spectrum of optic disk anomalies and diseases whose frequency correlates with the size of the optic disk. This spectrum includes abnormally small optic nerve heads with increased prevalence of drusen, pseudopapilledema and nonarteritic anterior ischemic optic neuropathy, asymptomatic macrodisks with physiologic, "pseudoglaucomatous" macrocups, symptomatic macrodisks with pits and, finally, optic nerve heads with morning glory syndrome. The last-mentioned may be considered an extreme variation of optic disks with pits which have combined to form a single macropit covering the entire optic nerve head.
The authors describe the ophthalmological findings and clinical course in two patients with EEC syndrome and one patient with anhydrotic ectodermal dysplasia. A retinal complication was seen in a 43-year-old patient which seems to be associated with the EEC syndrome and has not yet been described. A review of the literature indicates the frequency of ocular findings. So far, primary ocular changes in anhydrotic ectodermal dysplasia have never been observed. The older the patient, the more severe the secondary complications affecting the lids and cornea can be; the eye may even be lost.
The eyes of 18 adult Dachshunds of both sexes were examined histologically. Nine dogs were hornozygous for the Merle Factor (MM), five were heterozygotes (Mm) and four were normal (mm). The width of the ganglion cell layers as well as of the inner and outer nuclear layers was measured. There were statistically significant differences between the three groups of dogs. Half of the number of eyes from MM dogs showed severe pathological changes. The nuclear and ganglion cell layers were badly differentiated and retinal folds and rosettes were found. None of the homozygotes had a Tapetum lucidum (T. 1.) and only two of the heterozygotes had a T. 1. and this has in only one eye in each case. Retinal pigmentation was scanty in homozygotes as well as in heterozygotes. The remarkable resemblance of the Klein-Waardenburg syndrome of man and the Merle Factor of the dog is pointed out and the importance of the Merle Factor as an animal model is emphasized. For reasons of animal welfare it is necessary to restrict breeding with the Merle Factor to scientific purposes.
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